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Abnormal semicircular canal morphology

MedGen UID:
868972
•Concept ID:
C4023386
•
Anatomical Abnormality
Synonym: Morphological abnormality of the semicircular canal
 
HPO: HP:0011380

Definition

An abnormality of the morphology of the semicircular canal. [from HPO]

Conditions with this feature

Hearing loss, autosomal recessive 109
MedGen UID:
1633308
•Concept ID:
C4693935
•
Disease or Syndrome
DFNB109 is characterized by bilateral congenital severe to profound sensorineural hearing loss. In addition, affected individuals exhibit vestibular dysplasia on CT scan, although they do not manifest problems with balance or movement (Rohacek et al., 2017).
See: Condition Record
Hearing loss, autosomal dominant 80
MedGen UID:
1779667
•Concept ID:
C5543289
•
Disease or Syndrome
DFNA80 is characterized by nonsyndromic congenital deafness associated with absent or malformed cochleae and eighth cranial nerves (Schrauwen et al., 2018; Schrauwen et al., 2020).
See: Condition Record
Hearing loss, autosomal dominant 80
Hearing loss, autosomal recessive 109

Professional guidelines

PubMed

Cerebrospinal fluid otorrhea secondary to congenital inner ear dysplasia: diagnosis and management of 18 cases.
Wang B, Dai WJ, Cheng XT, Liuyang WY, Yuan YS, Dai CF, Shu YL, Chen B
J Zhejiang Univ Sci B 2019 Feb.;20(2):156-163. doi: 10.1631/jzus.B1800224. PMID: 30666849Free PMC Article
Endolymphatic hydrops imaging: Differential diagnosis in patients with Meniere disease symptoms.
Attyé A, Eliezer M, Galloux A, Pietras J, Tropres I, Schmerber S, Dumas G, Krainik A
Diagn Interv Imaging 2017 Oct;98(10):699-706. Epub 2017 Jun 20 doi: 10.1016/j.diii.2017.06.002. PMID: 28645678
Diagnosis and management of the patulous eustachian tube.
Poe DS
Otol Neurotol 2007 Aug;28(5):668-77. doi: 10.1097/mao.0b013e31804d4998. PMID: 17534202

Recent clinical studies

Etiology

Vestibular hydrops in patients with semicircular canal malformation.
Niu Y, Chen W, Lin M, Sha Y
Auris Nasus Larynx 2023 Oct;50(5):727-732. Epub 2023 Feb 4 doi: 10.1016/j.anl.2023.01.009. PMID: 36740469
Disorders of the inner-ear balance organs and their pathways.
Young AS, Rosengren SM, Welgampola MS
Handb Clin Neurol 2018;159:385-401. doi: 10.1016/B978-0-444-63916-5.00025-2. PMID: 30482329
Pediatric superior semicircular canal dehiscence: illustrative case and systematic review.
Lagman C, Ong V, Chung LK, Elhajjmoussa L, Fong C, Wang AC, Gopen Q, Yang I
J Neurosurg Pediatr 2017 Aug;20(2):196-203. Epub 2017 May 26 doi: 10.3171/2017.3.PEDS1734. PMID: 28548616
Meniere's disease.
Nakashima T, Pyykkö I, Arroll MA, Casselbrant ML, Foster CA, Manzoor NF, Megerian CA, Naganawa S, Young YH
Nat Rev Dis Primers 2016 May 12;2:16028. doi: 10.1038/nrdp.2016.28. PMID: 27170253
Inferior vestibular neuritis.
Kim JS, Kim HJ
J Neurol 2012 Aug;259(8):1553-60. Epub 2012 Jan 4 doi: 10.1007/s00415-011-6375-4. PMID: 22215238

Diagnosis

The spectrum of cochlear malformations in CHARGE syndrome and insights into the role of the CHD7 gene during embryogenesis of the inner ear.
Lewis MA, Juliano A, Robson C, Clement E, Nash R, Rajput K, D'Arco F
Neuroradiology 2023 Apr;65(4):819-834. Epub 2023 Jan 30 doi: 10.1007/s00234-023-03118-9. PMID: 36715725
Disorders of the inner-ear balance organs and their pathways.
Young AS, Rosengren SM, Welgampola MS
Handb Clin Neurol 2018;159:385-401. doi: 10.1016/B978-0-444-63916-5.00025-2. PMID: 30482329
Pediatric superior semicircular canal dehiscence: illustrative case and systematic review.
Lagman C, Ong V, Chung LK, Elhajjmoussa L, Fong C, Wang AC, Gopen Q, Yang I
J Neurosurg Pediatr 2017 Aug;20(2):196-203. Epub 2017 May 26 doi: 10.3171/2017.3.PEDS1734. PMID: 28548616
Meniere's disease.
Nakashima T, Pyykkö I, Arroll MA, Casselbrant ML, Foster CA, Manzoor NF, Megerian CA, Naganawa S, Young YH
Nat Rev Dis Primers 2016 May 12;2:16028. doi: 10.1038/nrdp.2016.28. PMID: 27170253
Inferior vestibular neuritis.
Kim JS, Kim HJ
J Neurol 2012 Aug;259(8):1553-60. Epub 2012 Jan 4 doi: 10.1007/s00415-011-6375-4. PMID: 22215238

Therapy

Vestibular hydrops in patients with semicircular canal malformation.
Niu Y, Chen W, Lin M, Sha Y
Auris Nasus Larynx 2023 Oct;50(5):727-732. Epub 2023 Feb 4 doi: 10.1016/j.anl.2023.01.009. PMID: 36740469
Anatomic Malformations of the Middle and Inner Ear in 22q11.2 Deletion Syndrome: Case Series and Literature Review.
Verheij E, Elden L, Crowley TB, Pameijer FA, Zackai EH, McDonald-McGinn DM, Thomeer HGXM
AJNR Am J Neuroradiol 2018 May;39(5):928-934. Epub 2018 Mar 15 doi: 10.3174/ajnr.A5588. PMID: 29545254Free PMC Article
Management of surgical difficulties during cochlear implant with inner ear anomalies.
Aldhafeeri AM, Alsanosi AA
Int J Pediatr Otorhinolaryngol 2017 Jan;92:45-49. Epub 2016 Nov 3 doi: 10.1016/j.ijporl.2016.11.001. PMID: 28012532
Meniere's disease.
Nakashima T, Pyykkö I, Arroll MA, Casselbrant ML, Foster CA, Manzoor NF, Megerian CA, Naganawa S, Young YH
Nat Rev Dis Primers 2016 May 12;2:16028. doi: 10.1038/nrdp.2016.28. PMID: 27170253
Otitic meningitis, superior semicircular canal dehiscence, and encephalocele: a case series.
Lim ZM, Friedland PL, Boeddinghaus R, Thompson A, Rodrigues SJ, Atlas M
Otol Neurotol 2012 Jun;33(4):610-2. doi: 10.1097/MAO.0b013e3182536de7. PMID: 22525216

Prognosis

Surgical management of endolymphatic sac tumor: classification, outcomes and strategy. A single institution's experience.
Wu N, Ma X, Shen W, Hou Z, Han W, Dai P, Zhao H, Huang D, Han D, Yang S
Eur Arch Otorhinolaryngol 2023 Jan;280(1):69-76. Epub 2022 Jun 23 doi: 10.1007/s00405-022-07447-y. PMID: 35739425
Otopathologic Abnormalities in CHARGE Syndrome.
da Costa Monsanto R, Knoll RM, de Oliveira Penido N, Song G, Santos F, Paparella MM, Cureoglu S
Otolaryngol Head Neck Surg 2022 Feb;166(2):363-372. Epub 2021 Apr 20 doi: 10.1177/01945998211008911. PMID: 33874787
Some Remarks on Imaging of the Inner Ear: Options and Limitations.
Giesemann A, Hofmann E
Clin Neuroradiol 2015 Oct;25 Suppl 2:197-203. Epub 2015 Jul 8 doi: 10.1007/s00062-015-0422-y. PMID: 26153464
Molecular genetic advances in semicircular canal abnormalities and sensorineural hearing loss: a report of 16 cases.
Yu KK, Mukherji S, Carrasco V, Pillsbury HC, Shores CG
Otolaryngol Head Neck Surg 2003 Dec;129(6):637-46. doi: 10.1016/S0194-59980301593-6. PMID: 14663429
Anatomic variations and anomalies involving the facial canal.
Nager GT, Proctor B
Otolaryngol Clin North Am 1991 Jun;24(3):531-53. PMID: 1762775

Clinical prediction guides

Vestibular hydrops in patients with semicircular canal malformation.
Niu Y, Chen W, Lin M, Sha Y
Auris Nasus Larynx 2023 Oct;50(5):727-732. Epub 2023 Feb 4 doi: 10.1016/j.anl.2023.01.009. PMID: 36740469
The spectrum of cochlear malformations in CHARGE syndrome and insights into the role of the CHD7 gene during embryogenesis of the inner ear.
Lewis MA, Juliano A, Robson C, Clement E, Nash R, Rajput K, D'Arco F
Neuroradiology 2023 Apr;65(4):819-834. Epub 2023 Jan 30 doi: 10.1007/s00234-023-03118-9. PMID: 36715725
Comparison of vestibular ocular reflex and gross motor development in children with semicircular canal aplasia and hypoplasia.
Kimura Y, Kaga K
Int J Pediatr Otorhinolaryngol 2022 Nov;162:111303. Epub 2022 Sep 15 doi: 10.1016/j.ijporl.2022.111303. PMID: 36137473
Pediatric superior semicircular canal dehiscence: illustrative case and systematic review.
Lagman C, Ong V, Chung LK, Elhajjmoussa L, Fong C, Wang AC, Gopen Q, Yang I
J Neurosurg Pediatr 2017 Aug;20(2):196-203. Epub 2017 May 26 doi: 10.3171/2017.3.PEDS1734. PMID: 28548616
Molecular genetic advances in semicircular canal abnormalities and sensorineural hearing loss: a report of 16 cases.
Yu KK, Mukherji S, Carrasco V, Pillsbury HC, Shores CG
Otolaryngol Head Neck Surg 2003 Dec;129(6):637-46. doi: 10.1016/S0194-59980301593-6. PMID: 14663429

Recent systematic reviews

The influence of the subarcuate artery in the superior semicircular canal dehiscence and its frequency on stillbirths: illustrative cases and systematic review.
Bom Braga GP, Noble JH, Gebrim EMMS, Labadie RF, Bento RF
Acta Otolaryngol 2018 May;138(5):437-442. Epub 2017 Dec 22 doi: 10.1080/00016489.2017.1416169. PMID: 29272988
Pediatric superior semicircular canal dehiscence: illustrative case and systematic review.
Lagman C, Ong V, Chung LK, Elhajjmoussa L, Fong C, Wang AC, Gopen Q, Yang I
J Neurosurg Pediatr 2017 Aug;20(2):196-203. Epub 2017 May 26 doi: 10.3171/2017.3.PEDS1734. PMID: 28548616

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